Use of Rituximab in child with SLE and myocardial involvement

A twelve year old girl of Pakistani origin first presented at the age of nine with weakness, lethargy, fever, and was diagnosed as SLE with progressive multisystem involvement with LV dysfunction, vasculitic skin lesions, hepatitis, hemiplegia and nephritis.

She was treated initially with Cyclophosphamide and intravenous steroids, and thereafter maintained on Methotrexate. She was also treated with Azathioprine and Mycophenolate mofetil as single agents for some time as she became non compliant with Methotrexate.

Three years into diagnosis she had symptoms of increasing tiredness, breathlessness and tachycardia. An echocardiogram confirmed ventricular dilatation suggesting myocardial involvement. She was recommenced on Cyclophosphamide with Methyprednisolone, and was also commenced on Frusemide and Enalapril which initiated control of her cardiac function. Her ejection fraction improved from 30% to 52%. Subsequently, after 6 months of Cyclophosphamide, Rituximab was tried as sole agent which is currently holding her lupus and myocardial changes in control for over 20 months. She has had 3 cycles of Rituximab, each given at 1 gram/kg, divided in two doses two weeks apart. Her latest ejection fraction is 45%.

There is very limited published evidence for Rituximab for cardiac involvement of SLE in children. Thus we would recommend that Rituximab be considered in this rare but potentially debilitating presentation of SLE for control as well as to limit the use of large doses of cytotoxic medications.